Harlequin ichthyosis (HI) is a very rare severe form of autosomal recessive congenital ichthyosis, usually associated with stillbirth and early neonatal death. A newborn girl with HI is described. She presented in a critical condition with severe universalis hyperkeratosis, diffuse scales and deep erythematous fissures. She received preventive systemic antibiotics and hygienic nursing with skin and eye care, feeding and appropriate hydration. She was discharged at 28 days in good general condition.
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